Pregnancy heart team: A lesion-specific approach.

Central MessageAlthough many women with repaired congenital heart disease can carry a pregnancy with low risk, management by an experienced pregnancy heart team is essential, and the surgeon has a critical role. Although many women with repaired congenital heart disease can carry a pregnancy with low risk, management by an experienced pregnancy heart team is essential, and the surgeon has a critical role. With 90% of children with congenital heart disease (CHD) surviving to adulthood,1Crumb S.R. Cook S.C. Cheatham J.P. Galantowicz M. Feltes T.F. Phillips A. et al.Quality outcomes of ACHD patients undergoing cardiovascular procedures and hospital admissions in a free-standing children's hospital.Int J Cardiol. 2011; 146: 326-329Abstract Full Text Full Text PDF PubMed Scopus (22) Google Scholar more females with CHD are living to childbearing age. Many of these women have varying degrees of residual lesions and/or altered physiology and require maintenance medications, with potential implications for pregnancy and lactation, as well as the possibility of further surgical interventions. Confronted with this burgeoning population approaching their reproductive years, cardiac surgeons must consider operative choices in the context of potential future pregnancies and also must be versed in situations warranting surgical intervention prior to conception and during pregnancy. The surgeon is a critical component of the pregnancy heart team. Women with cardiac diseases including CHD who become pregnant or are contemplating pregnancy should be managed by an experienced pregnancy heart team (Figure 1). This team involves specialists from adult congenital cardiology, cardiovascular imaging, interventional cardiology, high-risk obstetrics, genetics, and adult and/or congenital heart surgery, and in the setting of intervention and potential delivery, additional team members include neonatology, cardiac anesthesia, obstetric anesthesia, and perfusion specialists. Generally, this team is led by the cardiologist, with the patient having a critical role on the team and in decision-making. Given that these cases, and particularly those that require intervention, are less frequent within an institution, maintaining a consistent team to manage these patients is advantageous. Although many women with repaired CHD can safely carry a pregnancy with low risk, careful planning and management by an experienced multidisciplinary team both preconception (if possible) and during pregnancy are essential. This article focuses on the surgical considerations for women with CHD contemplating pregnancy as well as during pregnancy, and also briefly reviews the physiologic changes of pregnancy, describes risk stratification in the context of pregnancy, and highlights key issues pertinent to surgeons. During pregnancy, blood volume increases by ∼50%, comprising a 30% to 50% increase in plasma volume and a 20% to 30% increase in erythrocyte mass,2Gleicher N. Elkayam U. Principles and Practice of Medical Therapy in Pregnancy.2nd ed. Appleton & Lange, 1992Google Scholar resulting in a relative (dilutional) anemia. Other changes include decreases in systemic and pulmonary vascular resistance.3Connolly H.M. Pregnancy in women with congenital heart disease.Curr Cardiol Rep. 2005; 7: 305-309Crossref PubMed Scopus (10) Google Scholar The decrease in afterload during pregnancy has important sequela that includes regurgitant lesions generally are better tolerated than obstructive lesions.4Warnes C.A. Pregnancy and delivery in women with congenital heart disease.Circ J. 2015; 79: 1416-1421Crossref PubMed Scopus (37) Google Scholar There is also an increase in heart rate with an overall increase in cardiac output; this is a gradual process from the late first trimester until approximately 32 weeks of gestation, reaching a plateau that is 30% to 50% above preconception values. However, cardiac output increases still further during labor and delivery, to as much as 80% above the prepregnancy level.3Connolly H.M. Pregnancy in women with congenital heart disease.Curr Cardiol Rep. 2005; 7: 305-309Crossref PubMed Scopus (10) Google Scholar Inferior vena cava compression is relieved and venous return increases after delivery, along with myometrial involution, leading to an intravascular “autotransfusion.” This is followed by spontaneous diuresis, and hemodynamic parameters generally return to baseline by 2 to 4 weeks after vaginal delivery (longer after cesarean delivery).3Connolly H.M. Pregnancy in women with congenital heart disease.Curr Cardiol Rep. 2005; 7: 305-309Crossref PubMed Scopus (10) Google Scholar However, in some women with CHD, the increased volume load experienced during pregnancy can accelerate long-term cardiovascular decompensation.4Warnes C.A. Pregnancy and delivery in women with congenital heart disease.Circ J. 2015; 79: 1416-1421Crossref PubMed Scopus (37) Google Scholar Cyanotic CHD during pregnancy merits particular attention. Owing to the functional anemia, cyanosis will worsen, and the prothrombotic state during pregnancy increases the risk of thrombus formation in the pulmonary, leg, and pelvic vasculature.3Connolly H.M. Pregnancy in women with congenital heart disease.Curr Cardiol Rep. 2005; 7: 305-309Crossref PubMed Scopus (10) Google Scholar Cyanosis also increases the risk of spontaneous abortion and intrauterine growth retardation; in a large study of women with cyanotic heart disease, those with a resting oxygen saturation ≥90% had a 92% rate of live births, compared with 12% in those with saturation ≤85%.5Presbitero P. Somerville J. Stone S. Aruta E. Spiegelhalter D. Rabajoli F. Pregnancy in cyanotic congenital heart disease. Outcome of mother and fetus.Circulation. 1994; 89: 2673-2676Crossref PubMed Scopus (360) Google Scholar Preconception counseling includes comprehensive medication review to avoid teratogens. These include angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, and angiotensin receptor-neprilysin inhibitors (Figure 2).3Connolly H.M. Pregnancy in women with congenital heart disease.Curr Cardiol Rep. 2005; 7: 305-309Crossref PubMed Scopus (10) Google Scholar Beta-blockers are generally considered acceptable, although they (atenolol in particular) have been associated with impaired fetal growth, and thus patients requiring beta-blockers should receive serial obstetric sonographic monitoring.7Ommen S.R. Mital S. Burke M.A. Day S.M. Deswal A. Elliott P. et al.2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: a report of the American College of Cardiology/American Heart Association Joint Committee on clinical practice guidelines.Circulation. 2020; 142: e558-e631PubMed Google Scholar Most antiarrhythmic agents are relatively contraindicated in pregnancy.6Tamirisa K.P. Elkayam U. Briller J.E. Mason P.K. Pillarisetti J. Merchant F.M. et al.Arrhythmias in pregnancy.JACC Clin Electrophysiol. 2022; 8: 120-135Crossref PubMed Scopus (22) Google Scholar,7Ommen S.R. Mital S. Burke M.A. Day S.M. Deswal A. Elliott P. et al.2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: a report of the American College of Cardiology/American Heart Association Joint Committee on clinical practice guidelines.Circulation. 2020; 142: e558-e631PubMed Google Scholar For patients treated with warfarin anticoagulation, the risks and benefits must be weighed, and if anticoagulation is necessary consideration given to substituting either low molecular weight heparin (LMWH) or low-dose warfarin.7Ommen S.R. Mital S. Burke M.A. Day S.M. Deswal A. Elliott P. et al.2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: a report of the American College of Cardiology/American Heart Association Joint Committee on clinical practice guidelines.Circulation. 2020; 142: e558-e631PubMed Google Scholar Therapeutic anticoagulation is critical for patients with mechanical valve prostheses who decide to proceed with pregnancy and is discussed more completely below. A comprehensive review of anticoagulation during pregnancy can be found in the 2018 European Society of Cardiology guidelines.8Regitz-Zagrosek V. Roos-Hesselink J.W. Bauersachs J. Blomström-Lundqvist C. Cífková R. De Bonis M. et al.2018 ESC guidelines for the management of cardiovascular diseases during pregnancy.Eur Heart J. 2018; 39: 3165-3241Crossref PubMed Scopus (1087) Google Scholar Management of valve disease in young women approaching or in their childbearing years is particularly challenging. There are many occasions when successful valve repair is either not possible or not durable and replacement is the best option. Unfortunately, bioprosthetic valves have limited durability in the pediatric population, so many young women approach their childbearing years with a mechanical valve. Thorough counseling prior to a potential valve replacement should include discussion of the risks and benefits of a mechanical valve and particularly the challenges of managing anticoagulation during a planned or unplanned pregnancy. During pregnancy, there is a shift in the balance between procoagulant and anticoagulant factors, resulting in a net hypercoagulable state.9Steinberg Z.L. Dominguez-Islas C.P. Otto C.M. Stout K.K. Krieger E.V. Maternal and fetal outcomes of anticoagulation in pregnant women with mechanical heart valves.J Am Coll Cardiol. 2017; 69: 2681-2691Crossref PubMed Scopus (95) Google Scholar Warfarin, the standard therapy for anticoagulation for mechanical valves, has teratogenic effects during the sixth to ninth weeks of pregnancy (usually dose-related), rarely has been associated with fetal intracranial hemorrhage, and there are also reports of high rates of fetal loss.10Hall J.G. Pauli R.M. Wilson K.M. Maternal and fetal sequelae of anticoagulation during pregnancy.Am J Med. 1980; 68: 122-140Abstract Full Text PDF PubMed Scopus (767) Google Scholar, 11Iturbe-Alessio I. Fonseca M.C. Mutchinik O. Santos M.A. Zajarías A. Salazar E. Risks of anticoagulant therapy in pregnant women with artificial heart valves.N Engl J Med. 1986; 315: 1390-1393Crossref PubMed Scopus (261) Google Scholar, 12Sareli P. England M.J. Berk M.R. Marcus R.H. Epstein M. Driscoll J. et al.Maternal and fetal sequelae of anticoagulation during pregnancy in patients with mechanical heart valve prostheses.Am J Cardiol. 1989; 63: 1462-1465Abstract Full Text PDF PubMed Scopus (111) Google Scholar, 13Chan W.S. Anand S. Ginsberg J.S. Anticoagulation of pregnant women with mechanical heart valves: a systematic review of the literature.Arch Intern Med. 2000; 160: 191-196Crossref PubMed Scopus (632) Google Scholar Unfractionated heparin does not cross the placenta and thus has not been associated with deleterious effects on fetal development. Valve guidelines support the use of warfarin during the second and third trimesters, with transitioning to intravenous unfractionated heparin near term and cessation of heparin immediately prior to delivery.14Nishimura R.A. Otto C.M. Bonow R.O. Carabello B.A. Erwin III, J.P. Guyton R.A. et al.2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association task force on practice guidelines.Circulation. 2014; 129: e521-e643Crossref PubMed Scopus (1212) Google Scholar In the first trimester, continuation of warfarin is reasonable if the dose to attain therapeutic anticoagulation is ≤5 mg/day,14Nishimura R.A. Otto C.M. Bonow R.O. Carabello B.A. Erwin III, J.P. Guyton R.A. et al.2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association task force on practice guidelines.Circulation. 2014; 129: e521-e643Crossref PubMed Scopus (1212) Google Scholar given the teratogenic effect of <3% at those doses. For patients receiving higher warfarin doses, LMWH or intravenous unfractionated heparin is recommended given the >8% risk of warfarin affecting fetal development. Data on the risks and optimal management of anticoagulation for mechanical valves are limited. A meta-analysis comparing outcomes of warfarin throughout pregnancy, LMWH throughout pregnancy, LMWH in the first trimester and then warfarin, and heparin in the first trimester and then warfarin found the lowest composite maternal risk of death, thromboembolism, and valve failure of 5% with warfarin throughout pregnancy, compared to 16% for each of the other groups.9Steinberg Z.L. Dominguez-Islas C.P. Otto C.M. Stout K.K. Krieger E.V. Maternal and fetal outcomes of anticoagulation in pregnant women with mechanical heart valves.J Am Coll Cardiol. 2017; 69: 2681-2691Crossref PubMed Scopus (95) Google Scholar The composite fetal risk of spontaneous abortion, death, and congenital defects was lowest with LMWH throughout pregnancy at 13%, with risk for the other regimens ranging from 23% to 39%; however, no significant difference in fetal risk was observed for those receiving ≤5 mg/day of warfarin and LMWH.9Steinberg Z.L. Dominguez-Islas C.P. Otto C.M. Stout K.K. Krieger E.V. Maternal and fetal outcomes of anticoagulation in pregnant women with mechanical heart valves.J Am Coll Cardiol. 2017; 69: 2681-2691Crossref PubMed Scopus (95) Google Scholar Data from the Registry of Pregnancy and Cardiac Disease (ROPAC) reported a 1.4% maternal mortality for those with mechanical valves, matching the rate in those with bioprosthetic valves.15van Hagen I.M. Roos-Hesselink J.W. Ruys T.P. Merz W.M. Goland S. Gabriel H. et al.Pregnancy in women with a mechanical heart valve: data of the European Society of Cardiology Registry of Pregnancy and Cardiac Disease (ROPAC).Circulation. 2015; 132: 132-142Crossref PubMed Scopus (224) Google Scholar According to the ROPAC, valve thrombosis occurred in 5% (10 patients), 5 in the first trimester, all of whom had been switched to heparin.15van Hagen I.M. Roos-Hesselink J.W. Ruys T.P. Merz W.M. Goland S. Gabriel H. et al.Pregnancy in women with a mechanical heart valve: data of the European Society of Cardiology Registry of Pregnancy and Cardiac Disease (ROPAC).Circulation. 2015; 132: 132-142Crossref PubMed Scopus (224) Google Scholar Hemorrhagic events occurred in 23%, compared with 5% in patients with bioprosthetic heart valves. Warfarin was associated with higher rates of miscarriage (29% vs 9% for heparin) and late fetal death (7% vs 1%). Overall, only 58% had a pregnancy free of serious adverse events, compared with 79% of those with a bioprosthetic valve.15van Hagen I.M. Roos-Hesselink J.W. Ruys T.P. Merz W.M. Goland S. Gabriel H. et al.Pregnancy in women with a mechanical heart valve: data of the European Society of Cardiology Registry of Pregnancy and Cardiac Disease (ROPAC).Circulation. 2015; 132: 132-142Crossref PubMed Scopus (224) Google Scholar There are certain conditions for which pregnancy should be avoided for the safety of the mother, and if pregnancy occurs, termination should be considered. These conditions include severe pulmonary hypertension,16Avila W.S. Grinberg M. Snitcowsky R. Faccioli R. Da Luz P.L. Bellotti G. et al.Maternal and fetal outcome in pregnant women with Eisenmenger's syndrome.Eur Heart J. 1995; 16: 460-464Crossref PubMed Scopus (108) Google Scholar class III or IV congestive heart failure from systemic ventricular systolic dysfunction, severe obstructive lesions, vascular Ehlers–Danlos syndrome, single-ventricle (Fontan) circulations with concurrent organ dysfunction, an aortic root diameter of ≥45 mm in the setting of Marfan syndrome, and an aortic dimension of 50 mm for bicuspid aortic valve aortopathy without high-risk features and 40 mm for Loeys–Dietz syndrome. Historically, maternal mortality has been reported to be 25% to 30% for those with severe pulmonary hypertension, while a more recent study reported a rate of 12%.17Low T.T. Guron N. Ducas R. Yamamura K. Charla P. Granton J. et al.Pulmonary arterial hypertension in pregnancy-a systematic review of outcomes in the modern era.Pulm Circ. 2021; 11 (20458940211013671)Crossref PubMed Scopus (18) Google Scholar In the current era, with careful management, maternal mortality in patients with severe aortic stenosis should be close to 0%,18Orwat S. Diller G.P. van Hagen I.M. Schmidt R. Tobler D. Greutmann M. et al.Risk of pregnancy in moderate and severe aortic stenosis: from the multinational ROPAC registry.J Am Coll Cardiol. 2016; 68: 1727-1737Crossref PubMed Scopus (79) Google Scholar although hospitalization during pregnancy is common. Severe cyanosis is considered a relative contraindication to pregnancy, primarily because of the risks related to poor fetal outcome.5Presbitero P. Somerville J. Stone S. Aruta E. Spiegelhalter D. Rabajoli F. Pregnancy in cyanotic congenital heart disease. Outcome of mother and fetus.Circulation. 1994; 89: 2673-2676Crossref PubMed Scopus (360) Google Scholar The risk profile of pregnancy for each woman with CHD should be individually assessed by experts in this area, because there will be many women who do not fit into the above categories but whose complex CHD may make pregnancy ill-advised. Ideally, these discussions are carried out prior to conception. Surgeons should be aware of the algorithms that have been developed to help risk-stratify women with cardiac disease who become pregnant. Although these algorithms highlight major factors and provide a broad level of risk, each has limitations. Individual assessment by a specialty care team in experienced centers remains key in assessing the risk for a given patient. The most well-known risk stratification systems include CARPREG (Cardiac Disease in Pregnancy Study) I and II, ZAHARA (Zwangerschap bij Aangeboren HARtAfwijkingen; in English, Pregnancy in Women with Congenital Heart Disease), and maternal World Health Organization (mWHO). First published in 2001, the CARPREG score19Siu S.C. Sermer M. Colman J.M. Alvarez A.N. Mercier L.A. Morton B.C. et al.Cardiac Disease in Pregnancy (CARPREG) InvestigatorsProspective multicenter study of pregnancy outcomes in women with heart disease.Circulation. 2001; 104: 515-521Crossref PubMed Scopus (1068) Google Scholar was derived from a study of 562 pregnant women with both acquired heart disease and CHD, and predicted cardiovascular events based on factors such as prior cardiac events, New York Heart Association (NYHA) functional status, left heart obstruction, and ventricular systolic dysfunction.20Drenthen W. Boersma E. Balci A. Moons P. Roos-Hesselink J.W. Mulder B.J. et al.ZAHARA InvestigatorsPredictors of pregnancy complications in women with congenital heart disease.Eur Heart J. 2010; 31: 2124-2132Crossref PubMed Scopus (436) Google Scholar A revised second version of this score (CAPREG II)21Silversides C.K. Grewal J. Mason J. Sermer M. Kiess M. Rychel V. et al.Pregnancy outcomes in women with heart disease: the CARPREG II study.J Am Coll Cardiol. 2018; 71: 2419-2430Crossref PubMed Scopus (273) Google Scholar includes more specific parameters with individual weights. The ZAHARA risk stratification was developed based on the CARPREG data specifically for women with CHD.20Drenthen W. Boersma E. Balci A. Moons P. Roos-Hesselink J.W. Mulder B.J. et al.ZAHARA InvestigatorsPredictors of pregnancy complications in women with congenital heart disease.Eur Heart J. 2010; 31: 2124-2132Crossref PubMed Scopus (436) Google Scholar The mWHO classification assigns risk for women with CHD and acquired cardiac disease and is the recommended tool of the American Heart Association and American College of Cardiology (Figure 3).23American College of Obstetricians and Gynecologists' Presidential Task Force on Pregnancy and Heart Disease and Committee on Practice Bulletins—ObstetricsACOG practice bulletin No. 212: pregnancy and heart disease.Obstet Gynecol. 2019; 133: e320-e356Crossref PubMed Scopus (184) Google Scholar A study of 213 pregnancies found that these risk stratification systems tended to overestimate actual risk, with mWHO the most accurate, although still not very discriminatory.24Balci A. Sollie-Szarynska K.M. van der Bijl A.G. Ruys T.P. Mulder B.J. Roos-Hesselink J.W. et al.ZAHARA II investigatorsProspective validation and assessment of cardiovascular and offspring risk models for pregnant women with congenital heart disease.Heart. 2014; 100: 1373-1381Crossref PubMed Scopus (162) Google Scholar Analyses from the ROPAC did find that mWHO was strongly associated with adverse events.25Roos-Hesselink J.W. Ruys T.P. Stein J.I. Thilén U. Webb G.D. Niwa K. et al.ROPAC InvestigatorsOutcome of pregnancy in patients with structural or ischaemic heart disease: results of a registry of the European Society of Cardiology.Eur Heart J. 2013; 34: 657-665Crossref PubMed Scopus (311) Google Scholar In general, regurgitant lesions are better tolerated during pregnancy and might not necessitate intervention (Figure 4). Decision making is individualized and depends on the additional factors, such as symptomatology, results of exercise testing, ventricular dilation, systolic ventricular function, and other cardiac lesions. In addition, the feasibility of valve repair versus replacement also helps guide the decision of whether to repair prior to pregnancy or delay until after delivery. In contrast to regurgitant lesions, obstructive lesions (of any valve or vessel) are more problematic and usually require intervention prior to pregnancy. Anatomic factors determine whether a percutaneous approach is feasible (either native valve or valve-in-valve) or stenting in the case of aortic coarctation or pulmonary artery/conduit abnormalities. In the event that surgical valve replacement is needed, prosthesis selection is individualized. In the aortic position, the Ross procedure would be preferred, with bioprosthetic replacement the second-best option. In the pulmonary position, bioprostheses have demonstrated good durability. The best option in the mitral position is also a stented bioprosthesis, but durability is more limited, and eventual reoperation is inevitable despite the use of percutaneous valve-in-valve therapy as a bridge. The tricuspid valve lends itself to repair in most circumstances, but if replacement is necessary, porcine bioprostheses function well and have good durability in this age group. Mechanical prostheses in any position are usually avoided if pregnancy is planned, given the anticoagulation management is challenging, with potential complications for both the mother and fetus. Left ventricular outflow tract obstructive lesions require particular attention by surgeons and may require intervention prior to conception and rarely during pregnancy. Severe obstructive lesions are a contraindication for pregnancy. Obstructions to left ventricular outflow include aortic valvular stenosis, coarctation, subaortic stenosis, supravalvular aortic stenosis, and hypertrophic obstructive cardiomyopathy. Severe aortic stenosis is usually secondary to bicuspid or unicuspid valves in women of childbearing age. Aortic stenosis requires comprehensive evaluation preconception even if the patient is asymptomatic. An exercise test is recommended to assess exercise tolerance, symptoms, blood pressure response, heart rate recovery, and electrocardiographic changes. Patients with decreased resting left ventricular systolic function or an abnormal exercise test should not pursue pregnancy until valve repair or replacement has been performed.14Nishimura R.A. Otto C.M. Bonow R.O. Carabello B.A. Erwin III, J.P. Guyton R.A. et al.2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association task force on practice guidelines.Circulation. 2014; 129: e521-e643Crossref PubMed Scopus (1212) Google Scholar,26Regitz-Zagrosek V. Seeland U. Geibel-Zehender A. Gohlke-Bärwolf C. Kruck I. Schaefer C. Cardiovascular diseases in pregnancy.Dtsch Arztebl Int. 2011; 108: 267-273PubMed Google Scholar These patients also require cross-sectional imaging to evaluate for bicuspid valve–related aortopathy or associated coarctation. If the ascending aorta is >50 mm, aortic replacement should be considered prior to pregnancy.4Warnes C.A. Pregnancy and delivery in women with congenital heart disease.Circ J. 2015; 79: 1416-1421Crossref PubMed Scopus (37) Google Scholar If aortic valve replacement is planned, then the threshold is 45 mm. Of note, those with aortic stenosis and a moderate degree of obstruction prior to pregnancy also can experience symptomatic deterioration during pregnancy. For women with aortic stenosis who do become pregnant, the physiologic changes of pregnancy (eg, reduced systemic vascular resistance, higher cardiac output) increase the gradient and can result in angina, heart failure, syncope, and arrhythmias.27Silversides C.K. Colman J.M. Sermer M. Farine D. Siu S.C. Early and intermediate-term outcomes of pregnancy with congenital aortic stenosis.Am J Cardiol. 2003; 91: 1386-1389Abstract Full Text Full Text PDF PubMed Scopus (172) Google Scholar Additionally, cardiac output reserve in these patients is relatively impaired, which limits perfusion to the uterus and can lead to intrauterine growth retardation, preterm birth, and low birth weight.4Warnes C.A. Pregnancy and delivery in women with congenital heart disease.Circ J. 2015; 79: 1416-1421Crossref PubMed Scopus (37) Google Scholar Depending on the situation, medical treatment with a beta-blocker to decrease the heart rate, especially if the left ventricle is hyperdynamic or hypertrophied, can be helpful.4Warnes C.A. Pregnancy and delivery in women with congenital heart disease.Circ J. 2015; 79: 1416-1421Crossref PubMed Scopus (37) Google Scholar If heart failure develops, cautious use of a diuretic can reduce circulatory overload. When feasible, percutaneous balloon valvuloplasty has been successfully performed with lead shielding the fetus and should be applied in the setting of a pliable, noncalcified aortic valve with minimal regurgitation and surgical backup.28Vitale N. De Feo M. De Santo L.S. Pollice A. Tedesco N. Cotrufo M. Dose-dependent fetal complications of warfarin in pregnant women with mechanical heart valves.J Am Coll Cardiol. 1999; 33: 1637-1641Crossref PubMed Scopus (403) Google Scholar For those who remain symptomatic despite medical therapy, antepartum valve replacement should be considered. Depending on gestational age, this can be performed concomitantly with cesarean section delivery.29John A.S. Gurley F. Schaff H.V. Warnes C.A. Phillips S.D. Arendt K.W. et al.Cardiopulmonary bypass during pregnancy.Ann Thorac Surg. 2011; 91: 1191-1196Abstract Full Text Full Text PDF PubMed Scopus (125) Google Scholar There are also limited reports regarding the success of transcatheter aortic valve replacement during pregnancy.30Elkayam U. Bansal P. Mehra A. Catheter-based interventions for the management of valvular heart disease during pregnancy.JACC Adv. 2022; 1: 1-20Crossref Google Scholar Subvalvar (such as subaortic membrane) and supravalvular aortic stenosis are handled similarly to aortic stenosis in terms of indications for surgery, with the exception that catheter-based options are not effective. Coarctation carries a risk of impaired blood supply to the fetus with subsequent risk of compromised fetal growth and is also associated with risks to the mother. Many of these patients have already undergone interventions, and their prognosis depends on the degree of residual or recurrent coarctation. These patients commonly also have a bicuspid aortic valve and/or Shone syndrome and require evaluation for those associated lesions. There is also a slightly increased risk of aortic dissection secondary to both the increased cardiac output and the hormonal changes with pregnancy.4Warnes C.A. Pregnancy and delivery in women with congenital heart disease.Circ J. 2015; 79: 1416-1421Crossref PubMed Scopus (37) Google Scholar Interventions during pregnancy are generally limited to cases of aortic dissection, uncontrollable hypertension, or heart failure. Although percutaneous intervention may be considered, there may be an increased risk of dissection during pregnancy.4Warnes C.A. Pregnancy and delivery in women with congenital heart disease.Circ J. 2015; 79: 1416-1421Crossref PubMed Scopus (37) Google Scholar,31Ciresi C.M. Patel P.R. Asdell S.M. Hopkins K.A. Hoyer M.H. Kay W.A. Management of severe coarctation of the aorta during pregnancy.JACC Case Rep. 2020; 2: 116-119Crossref PubMed Google Scholar In general, pregnancy is well tolerated in patients with obstructive hypertrophic cardiomyopathy (HCM), with only 3 maternal deaths reported in the literature.7Ommen S.R. Mital S. Burke M.A. Day S.M. Deswal A. Elliott P. et al.2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: a report of the American College of Cardiology/American Heart Association Joint Committee on clinical practice guidelines.Circulation. 2020; 142: e558-e631PubMed Google Scholar There have not been any reported differences in outcomes for women with obstructive HCM and those with nonobstructive HCM. One-quarter of women report symptoms during pregnancy, with the majority having been symptomatic prior to pregnancy.7Ommen S.R. Mital S. Burke M.A. Day S.M. Deswal A. Elliott P. et al.2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: a report of the American College of Cardiology/American Heart Association Joint Committee on clinical practice guidelines.Circulation. 2020; 142: e558-e631PubMed Google Scholar Concomitant arrhythmias, such as atrial fibrillation, are managed as in other pregnant women. Beta-blockers are used as needed and should be continued when initiated prior to pregnancy.7Ommen S.R. Mital S